Epidemiologic Features of Systemic Vasculitides in the Southeast Region of Brazil

Abstract

Background/Objective The epidemiology of vasculitis is variable in different geographic areas, and this issue has not been approached in Brazil yet. The objective of this study was to assess the frequency of vasculitis in specialized centers in Brazil. Methods This cross-sectional study was performed in 9 vasculitis outpatient clinics from 6 different states mainly from the Southeast and the Northeast regions of Brazil between 2015 and 2017. Diagnosis and/or classification criteria for Behçet disease (BD), Takayasu arteritis (TA), giant cell arteritis (GCA), polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic granulomatosis with polyangiitis (EGPA), and cryoglobulinemic vasculitis (CryoVas) were used to include patients with at least 6 months of follow-up in this hospital-based survey. Results A total of 1233 patients with systemic vasculitis were included from the Southeast region. Behçet disease was the most frequent vasculitis (35.0%) followed by TA (26.4%), GPA (16.2%), PAN (5.8%), GCA (5.8%), EGPA (4.3%), MPA (3.4%), and CryoVas (3.0%). Up to 7.8% of vasculitis patients had a juvenile onset, and the frequency of vasculitides found in children and adolescents was as follows: TA (52.6%), BD (24.7%), GPA (12.4%), and PAN (10.3%). No cases of EGPA, MPA, and CryoVas were diagnosed before the age of 18 years. As a comparator, 103 vasculitis patients were included in the Northeast of Brazil where TA was found in 36.9% and BD in 31.1% of vasculitis cases. No GCA cases were found in the Northeast part of Brazil. Conclusions Similar to the epidemiology of vasculitis in Asia, BD and TA are the most frequent vasculitis in Southeastern Brazilian referral centers.

Increased modifiable cardiovascular risk factors in patients with Takayasu arteritis: a multicenter cross-sectional study

“…In the present study, only women were selected due to the higher prevalence of this gender in TAK [1,2]. Individuals over 50 years of age were excluded to minimize the inclusion of individuals with comorbidities inherent in older age groups, as well as patients with atherosclerosis and peripheral arterial disease [41], or other vasculitis such as giant cell arteritis [42].…”

“…Takayasu arteritis (TAK) is a primary systemic vasculitis characterized mainly by large-diameter vessels involvement, such as the aorta and major branches. Epidemiological studies have shown that the disease mainly affects women under the age of 40 years of age [1,2].…”

Endovascular treatment in Behçet’s disease: an integrative review

“…However, it cannot be classified solely according to the caliber of the vessels involved, since it can affect small, medium, and large caliber vessels, in contrast to other types of systemic vasculitis, such as Takayasu's arteritis and giant-cell arteritis (which have a predilection for largecaliber vessels), polyarteritis nodosa and Kawasaki disease (with predilection for medium-caliber vessels), or antineutrophil cytoplasmic antibodies vasculitis (with a preference for small-caliber vessels). 9 The vascular form of the disease, vasculo-Behçet, is present in around 7 to 38% of cases and venous involvement is more common than arterial. 3,10 Arterial injuries have prevalence ranging from 3.6 to 31%, 3 generally develop in the aorta and pulmonary artery and their main branches, 11 and can vary from aneurysms to acute arterial thromboses or stenoses, most often affecting the renal, pulmonary, and abdominal arteries.…”

“…The fact that it presents with nonspecific initial manifestations means that it may not by recognized by pediatricians and therefore recurrent oral ulcers at any phase of childhood or adolescence should be taken as a warning of the possibility of BD. 17 The disease has a very distinct geographical distribution, occurring predominantly in regions along the ancient "Silk Road", 9 running between Asia and the Mediterranean Basin, and it therefore affects many inhabitants of Japan, Iran, and Turkey. Prevalence is low in Western countries.…”

“…There are few epidemiological data on vasculites in Brazil, but those that are available suggest that BD is the country's most common systemic vasculitis, followed by Takayasu's Arteritis. 9 There is no universally accepted test to diagnose BD, so diagnosis is made clinically, on the basis of its classic triad of signs: oral ulcers, genital ulcers, and uveitis. 3 Symptoms occur in a pattern of unexpected exacerbations and remissions and the frequency of episodes reduces over time.…”